Haploidentical Transplant in Radiosensitive Severe Combined Immunodeficiency Disease.

Severe combined immunodeficiency (SCID) is an inborn error of immunity invariably resulting in mortality in infancy until managed by hematopoietic stem cell transplant (HSCT). We present an unusual case of SCID with a rare mutation involving the non-homologous end-joining 1 (NHEJ1) gene, where a haploidentical HSCT was carried out with modified conditioning and graft versus host prophylaxis regimen using proteasome inhibitor bortezomib with a successful outcome.

Non-local triple quantum dot thermometer based on Coulomb-coupled systems.

Recent proposals towards non-local thermoelectric voltage-based thermometry, in the conventional dual quantum dot set-up, demand an asymmetric step-like system-to-reservoir coupling around the ground states for optimal operation (Physica E, 114, 113635, 2019). In addition to such demand for unrealistic coupling, the sensitivity in such a strategy also depends on the average measurement terminal temperature, which may result in erroneous temperature assessment. In this paper, we propose non-local current based thermometry in the dual dot set-up as a practical alternative and demonstrate that in the regime of high bias, the sensitivity remains robust against fluctuations of the measurement terminal temperature.